P1457: MOTION ABNORMALITIES IN THE LEFT VENTRICLE OF THALASSEMIA INTERMEDIA PATIENTS
Authors: Antonella Meloni, Laura Pistoia, Vincenza Rossi, Maria Grazia Roberti, Luciana Rigoli, Stefano Pulini, Sabrina Armari, Gaetano Roccamo, Gennaro Restaino, Riccardo Righi, Vincenzo Positano, Filippo Cademartiri
Journal: HemaSphere (2023)

Topic: 27. Thalassemias Background: Motion abnormalities of the left ventricle (LV) have been reported in thalassemia major patients but no specific data is available for thalassemia intermedia (TI) patients. Motion abnormalities can be detected through a qualitative analysis of cine magnetic resonance (MR) images. Moreover, MR is the non-invasive gold standard technique for the quantification of myocardial iron overload (MIO) and biventricular function parameters and for the detection of myocardial fibrosis. Aims: We investigated the MR imaging and clinical correlates of LV motion abnormalities in a relatively large cohort of TI patients. Methods: MR was performed in 254 beta-TI patients (43.14±13.69 years, 138 females), consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia project. Cine images were acquired to evaluate wall motion and to quantify LV volumes, mass, and ejection fraction (EF). For MIO assessment, a T2* multislice approach was used. To detect replacement myocardial fibrosis, late gadolinium enhanced (LGE) images were acquired. During image analysis the 17-segment LV model of the standard AHA/ACC was taken into account. On the cine images, segmental wall motion was visually assessed and scored as 1=normal, 2=hypokinesia, 3=akinesia and 4=dyskinesia. The T2* value in all segments as well as the global value were calculated. Presence/absence of enhancing area was assessed for each segment. Results: Abnormal motion of the LV was found in 21 (8.3%) patients: 13 were hypokinetic while 8 were dyskinetic. There was a predominant involvement of wall motion abnormalities in the septal segments. The Table shows the comparison between patients with normal and abnormal LV wall motion. No difference in terms of age, gender, and cardiac iron levels was detected. LV volumes and mass indexed by body surface area were significantly higher in patients with abnormal LV motion while the LV EF was significantly lower. The contrast medium was administrated in 103 (40.6%) patients, and replacement myocardial fibrosis was detected in 27 (26.2%) of them. Two patients showed an ischemic pattern while the remaining 25 patients had a non-ischemic pattern of myocardial fibrosis, involving the septum in the 80.0% of cases. There was a significant association between the presence of enhancement and motion abnormalities. - Patients with normal wall motion (N=233) Patients with wall motion abnormalities (N=21) P-value Age (years ) 42.78±13.89 47.15±10.56 0.138 Female sex, N (% ) 130 (55.8) 8 (38.1) 0.119 Regular transfusions, N (% ) 143 (61.4) 11 (52.4) 0.419 Global Heart T2* (ms ) 40.79±6.01 38.71±7.19 0.252 N. of segments with T2* <20 ms 0.32±1.55 0.62±2.39 0.486 Global heart T2* <20 ms, N (% ) 4 (1.7) 1 (4.8) 0.353 LV end-diastolic volume index (ml/m 2 ) 88.59±19.37 98.76±16.14 0.020 LV end-systolic volume index (ml/m 2 ) 33.16±11.77 43.76±14.61 0.001 LV mass index (g/m 2 ) 58.16±14.22 67.62±18.46 0.029 LV ejection fraction (% ) 63.16±6.86 57.90±7.79 0.001 LGE, N (% ) 21/93 (22.6) 6/10 (60.0) 0.019 Summary/Conclusion: Movement abnormalities in the left ventricle were not really frequent in TI patients but were associated LV remodeling and dysfunction and with replacement myocardial fibrosis. Therefore, they may be a reflection of early myocardial damage, independent from cardiac iron accumulation. Keywords: Magnetic resonance imaging, Thalassemia